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Metabolic acidosis
Ammonia intoxication
Carbamoyl-P synthetase I
Urea cycle defect
Vit B12 and folate
H4-biopterin
1
Metabolic acidosis (p. 681)
Kidney extracts little Gln from bloodstream normally
Acidosis increases glutamine processing in kidney
NH4+ + metabolic acids salts (excreted in urine)
-ketoglutarate bicarbonate (HCO3-, buffer)
In kidney
Gln
TCA
cycle (buffer)
-ketoglutarate HCO3-
kidneys +
mitochondria NH4+ + acids Salts
(excreted)
Glutamate
dehydrogenase
Symptoms
Coma
Cerebral edema
Increase cranial pressure
Possible mechanisms
Depletion of ATP in brain cells
Changes of cellular osmotic balance in brain
Depletion of neurotransmitter
Remove excess NH4+
Glutamate dehydrogenase: NH4+ + -KG Glu
Glutamine synthetase: NH4+ + Glu Gln
[Glu] [GABA]
H2 biopterin
reductase